Abstract Neuroblastoma (NB) is a solid tumor of the sympathetic nervous system, most commonly found in childhood, standing for 7% of all pediatric malignancies. The incidence in adults is markedly smaller: 1 case per 10 million adults per year. We report the case of a previously healthy 27-year-old woman who started with lumbar pain, asthenia, and abdominal distension over the last month. A chest and abdomen tomography scan showed a huge mass in the upper left hemithorax and marked hepatomegaly. The diagnosis was confirmed by hepatic and lung biopsies. On day 4, after admission, the patient started chemotherapy. On the following days, she had severe vaginal bleeding, epistaxis, worsening of the hepatic function markers, refractory shock, and multiple organ dysfunction. She died on the twelfth day of admission. We also present a review of adult cases of NB reported in the past 5 years.

Abstract: Investigation of hyperferritinemia in metabolic syndrome patients represents a diagnostic challenge, but it is essential for the identification of individuals with iron overload. Hepcidin negatively regulates iron absorption and release. An increase in hepcidin occurs when iron levels are sufficient or in inflammatory states, conditions often associated with hyperferritinemia. Hemochromatosis causes hyperferritinemia due to iron overload, but frequently has low hepcidin levels. Our aim was to evaluate biochemical and molecular parameters related to iron metabolism in patients with metabolic syndrome. We evaluated 94 patients with metabolic syndrome according to the International Diabetes Federation criteria in a cross-sectional study. Anthropometric data and diagnostic criteria for metabolic syndrome, iron dosage, ferritin, transferrin saturation, hepcidin, and the C282Y and H63D mutations in the HFE hemochromatosis gene were evaluated. Prevalence of hyperferritinemia in the study population was 27.7% and was higher in males (46.2%) than in females (14.5%). Increase in transferrin saturation correlated with mutations in the hemochromatosis gene. Hyperferritinemia was associated to transferrin saturation and hepcidin after logistic regression analysis. In conclusion, hyperferritinemia is a frequent finding in metabolic syndrome patients, most frequently in men; and hepcidin assessment can be useful for the investigation of ferritin increase in those subjects.

RESUMO OBJETIVO: Ki-67 é uma proteína nuclear associada à proliferação celular em condições normais ou leucêmicas que pode ajudar a Identificar doenças mais agressivas. Este marcador é geralmente avaliado com imuno-histoquímica. O objetivo deste estudo foi avaliar a expressão de Ki-67 em amostras de neoplasias de células B maduras com imunofenotipagem por citometria de fluxo. MÉTODO: Após marcação de superfície com CD19 e CD45, foi realizada marcação intracelular para Ki-67 em células B maduras leucémicas. A expressão de Ki-67 foi avaliada por citometria de fluxo. RESULTADOS: A expressão de Ki-67 foi maior em células de linfomas de manto, linfoma de Burkitt e linfoma difuso de grandes células B. Também houve associação de Ki-67 à intensidade de fluorescência média de CD38. CONCLUSÃO: A expressão de Ki-67 avaliada por citometria de fluxo pode ser útil no diagnóstico de neoplasias de células B maduras. São necessários mais estudos para validar a avaliação de Ki-67 com Imunofenotipagem por citometria de fluxo.

SUMMARY OBJECTIVE: Ki-67 is a nuclear protein associated with cellular proliferation in normal or leukemic conditions that can help identify more aggressive diseases and is usually evaluated with immunohistochemistry. The aim of this was to assess Ki-67 expression on mature B-cell neoplasms samples with flow cytometry immunophenotyping. METHOD: After surface staining with CD19 and CD45, intracellular staining for Ki-67 was performed in leukemic mature B-cells. Ki-67 expression was evaluated with flow cytometry. RESULTS: Ki-67 expression was higher in mantle cell lymphoma, Burkitt lymphoma, and diffuse large B-cell lymphoma cases. It was also associated with CD38 mean fluorescence intensity. CONCLUSIONS: Ki-67 expression evaluated by flow cytometry can be a useful tool in the diagnosis of mature B-cell neoplasms. More studies are needed to validate Ki-67 assessment with flow cytometry immunophenotyping.

Abstract Background Distinction between mature B-cell neoplasms can be difficult due to overlapping of immunologic features and clinical manifestations. This study investigated whether quantifying mean fluorescence intensity of four monoclonal antibodies in a flow cytometry panel is useful for the differential diagnosis and characterization of these disorders. Methods The expressions of CD52, CD200, CD123 and CD43 were analyzed in samples from 124 patients with mature B-cell neoplasms. The quantitative estimation of these antigens was assessed by mean fluorescence intensity. Results The cases included were 78 chronic lymphocytic leukemias, three atypical chronic lymphocytic leukemias, six marginal zone lymphomas, 11 splenic marginal zone lymphomas, nine lymphoplasmacytic lymphomas, six mantle cell lymphomas, two hairy cell leukemias, two hairy cell leukemias variant, five follicular lymphomas, one Burkitt lymphoma and one diffuse large B-cell lymphoma. The mean fluorescence intensity of CD200 was higher in atypical chronic lymphocytic leukemia, chronic lymphocytic leukemia and hairy cell leukemia cases. CD123 showed higher mean fluorescence intensities in hairy cell leukemia cells. Chronic lymphocytic leukemia, atypical chronic lymphocytic leukemia and mantle cell lymphoma had higher expression of CD43 and all follicular lymphoma cases had very low mean fluorescence intensity values. CD52 expression was consistently positive among all cases. Conclusion Quantitative evaluation of these markers can be a useful additional tool to better identify some types of mature B-cell neoplasms.

Na região Sul do Brasil, a partir de 1989, foram descritos inúmeros casos de contato acidental com o corpo de lagartas venenosas da mariposa da espécie Lonomia obliqua Walker, 1855 (Lepidoptera: Saturniidae). As lagartas de L. obliqua possuem comportamento gregário e alimentam- -se de folhas das árvores hospedeiras durante a noite, permanecendo agrupadas no tronco durante o dia, o que favorece a ocorrência de acidentes. Essa lagarta possui o corpo recoberto por cerdas urticantes, que, ao contato com a pele dos indivíduos, se rompem e liberam seu conteúdo, inoculando o veneno na vítima. A constituição básica do veneno é proteica e seus componentes produzem modificações fisiológicas no acidentado, que incluem distúrbios na hemostasia. Síndrome hemorrágica associada a coagulopatia de consumo, hemólise intravascular e insuficiência renal aguda são algumas das manifestações clínicas possíveis relacionadas ao envenenamento por L. obliqua. Ainda não foram descritas avaliações laboratoriais específicas para diagnóstico do envenenamento, que é feito com base na anamnese do paciente, nas manifestações clínicas, nos índices hematimétricos e, principalmente, nos parâmetros que avaliam a coagulação sanguínea. A terapêutica é feita com a utilização de medidas de suporte e com a administração de um soro heterólogo hiperimune específico. Os envenenamentos podem ser graves e até mesmo fatais.

Summary In southern Brazil, since 1989, several cases of accidents produced by unwilling contact with the body of poisonous caterpillars of the moth species Lonomia obliqua Walker, 1855 (Lepidoptera: Saturniidae), were described. L. obliqua caterpillars have gregarious behavior and feed on leaves of host trees during the night, staying grouped in the trunk during the day, which favors the occurrence of accidents with the species. This caterpillar has the body covered with bristles that on contact with the skin of individuals, breaks and release their contents, inoculating the venom into the victim. The basic constitution of the venom is protein and its components produce physiological changes in the victim, which include disturbances in hemostasis. Hemorrhagic syndrome associated with consumption coagulopathy, intravascular hemolysis and acute renal failure are some of the possible clinical manifestations related to poisoning by L. obliqua. Specific laboratory tests for diagnosis of poisoning have not been described previously. The diagnosis of poisoning is made based on the patient's medical history, clinical manifestations, erythrocyte levels, and, primarily, parameters that evaluate blood coagulation. Treatment is performed with the use of supportive care and the administration of specific hyperimmune antivenom. Poisoning can be serious and even fatal.

Objetivo Determinar a prevalência de massa óssea baixa em pacientes adolescentes e adultos com fibrose cística e estudar os fatores potencialmente associados. Métodos Densidade mineral óssea foi determinada por absorciometria por dupla emissão de raios X na coluna lombar em pacientes ≤ 19 anos e na coluna e no fêmur em pacientes ≥ 20 anos. Avaliações nutricionais, bioquímicas e pulmonares foram realizadas. Dados referentes ao tratamento farmacológico foram coletados. Resultados 58 pacientes foram incluídos no estudo (25 homens/33 mulheres), média de idade de 23,9 anos (16-53). Massa óssea abaixo da esperada foi verificada em 20,7% dos pacientes. Não houve histórico de fratura. Z-score da coluna lombar associou-se positivamente com índice de massa corporal (r=0,3; p=0,022), volume expiratório forçado (% previsto) (r=0,415; p=0,001). A média do Z-score da coluna foi mais alta nas mulheres que nos homens (p=0,001), em pacientes que não possuíam insuficiência pancreática (p=0,02) e em pacientes que não haviam sido hospitalizados nos últimos três meses (p=0,032). Os fatores encontrados como preditores independentes de Z-score da coluna lombar foram sexo masculino (p=0,001) e índice de massa corporal (p=0,001). Conclusão Massa óssea baixa é frequente em pacientes com FC, estando associada independentemente com índice de massa corporal e sexo masculino.

Objective The aim of this cross-sectional study was to evaluate the prevalence of low bone mass density in cystic fibrosis patients as well as to evaluate the factors associated with bone mass in such patients. Methods Bone mass density was measured by dual-photon X-ray absorptiometry of lumbar spine (L1-L4), in patients ≤19 years old, or lumbar spine and femur (total and neck) in patients ≥20 years old. Evaluations of nutritional status, biochemical parameters, and lung function were performed. Medication data were obtained from medical records. Results Fifty-eight patients were included in the study (25 males/ 33 females), mean age 23.9 years (16-53years). The prevalence of bone mass below the expected range for age at any site was 20.7%. None of the subjects had history of fracture. Lumbar spine Z-score in cystic fibrosis patients correlated positively with body mass index (r= 0.3, p=0.001), and forced expiratory volume in the first second (% predicted) (r=0.415, p=0.022). Mean lumbar spine Z-score was higher in women (p=0.001), in patients with no pancreatic insufficiency (p=0.032), and in patients with no hospitalization in the last 3 months (p=0.02). After multivariate analysis, body mass index (p= 0.001) and sex (p=0.001) were independently associated with Z-score in lumbar spine. Conclusion Low bone mass is a frequent problem in patients with CF, being independently associated with body mass index, and male sex.

OBJETIVO: O objetivo desse estudo foi verificar a associação do nível sérico do magnésio com o uso de inibidores de bomba de prótons (IBP) e outros fatores. MÉTODOS: Realizou-se estudo transversal com 151 pacientes admitidos com doenças agudas no serviço de medicina interna do Hospital de Clínicas de Porto Alegre. Foram excluídos aqueles pacientes com condições usualmente relacionadas à hipomagnesemia: diarréia; vômitos; diabéticos agudamente descompensados; uso crônico de laxantes, álcool, diuréticos ou outros fármacos relacionados. RESULTADOS: Todos os pacientes apresentaram níveis normais de magnésio. Albumina e creatinina sérica se associaram positivamente com os níveis de magnésio sérico, após ajuste para fatores confundidores. Não houve diferença no nível sérico de magnésio em usuários ou não-usuários de IBP ou entre homens e mulheres. Não houve correlação com idade, nível sérico de fósforo e potássio. As principais limitações desse estudo foram a ausência de instrumento para medir a adesão aos IBPs e o tamanho da amostra. CONCLUSÃO: A associação do uso de IBP e hipomagnesemia é rara. Defeitos congênitos no metabolismo do magnésio devem ser responsáveis pelo surgimento de hipomagnesemia em usuários de dessa classe de fármacos.

OBJECTIVE: The aim of this study was to evaluate the association of serum magnesium levels with proton pump inhibitors (PPIs) use and other factors. METHODS: This was a cross-sectional study of 151 patients admitted with acute diseases in the Internal Medicine Division of the Hospital de Clinicas de Porto Alegre, after the exclusion of conditions that are commonly associated with hypomagnesemia: diarrhea; vomiting; chronic alcohol use; severely uncompensated diabetes mellitus; and chronic use of laxatives, diuretics or other drugs causing magnesium deficiency. RESULTS: All patients had normal serum magnesium levels. Serum albumin and creatinine levels were positively associated with serum magnesium levels, after adjusting for confounders. There was no difference between mean serum magnesium levels of PPI users and non-users, nor between men and women; there was also no correlation among age, serum phosphorus, and potassium levels with serum magnesium levels. Limitations of this study include the absence of an instrument for measuring adherence to PPI use and the sample size. CONCLUSION: The association of PPI use and hypomagnesemia is uncommon. Congenital defects in the metabolism of magnesium may be responsible for hypomagnesemia in some patients using this drug class.

OBJETIVO: O objetivo deste estudo foi avaliar a prevalência de deficiência de vitamina B12 em pacientes diabéticos brasileiros tratados com metformina e os fatores associados aos níveis séricos de vitamina B12. MÉTODO: Corte transversal. RESULTADOS: 144 pacientes foram incluídos. Os níveis séricos de vitamina B12 foram baixos (< 125 pmol/L) em 10 pacientes (6,9%) e possivelmente baixos (125 - 250pmol/L) em 53 pacientes (36,8%). Os níveis séricos de vitamina B12 foram associados negativamente à idade (B = -3,17; β = -0,171; p = 0,037) e à duração do uso de metformina (B = -33,36; β= -0,161; p = 0,048) e positivamente com a ingestão estimada de vitamina B12 (B = 67,96; β = 0,249; p = 0,002). CONCLUSÃO: Estes resultados sugerem alta prevalência de deficiência de vitamina B12 em pacientes diabéticos tratados com metformina. Pacientes mais velhos, em uso de metformina há muito tempo e com ingestão baixa de vitamina B12 estão provavelmente mais predispostos a essa deficiência.

OBJECTIVE: The objective of this study was to evaluate the presence of vitamin B12 deficiency and the factors associated with serum vitamin B12 levels in a sample of metformin-treated Brazilian diabetic patients. METHOD: Cross-sectional study. RESULTS: 144 patients were included. Serum vitamin B12 levels were low (< 125 pmol/L) in 10 patients (6.9%) and possibly low (125 - 250 pmol/L) in 53 patients (36.8%). Serum vitamin B12 levels were negatively associated with age (B = -3.17; β= -0.171; p = 0.037) and duration of metformin use (B= -33.36; β= -0.161; p = 0.048), and positively associated with the estimated intake of vitamin B12 (B= 67.96; β= 0.249; p = 0.002). CONCLUSION: The present findings suggest a high prevalence of vitamin B12 deficiency in metformin-treated diabetic patients. Older patients, patients in long term treatment with metformin and low vitamin B12 intake are probably more prone to this deficiency.

O uso de hemocomponentes em hospitais de alta complexidade é elevado, sendo necessário controle e racionalização de sua utilização, dada sua escassez e alto custo. Para tal, muitos hospitais desenvolveram protocolos para uso de hemocomponentes. O objetivo deste trabalho foi descrever as características epidemiológicas das solicitações de hemoderivados e sua adequação em conformidade ao protocolo vigente no Hospital de Clínicas de Porto Alegre. Por meio de estudo retrospectivo transversal, foram avaliadas a freqüência de transfusões e suas indicações no ano de 2005 em três áreas (Internação Clínica, Cirúrgica e Centro de Terapia Intensiva). Analisaram-se as justificativas de cada solicitação em conformidade com o protocolo vigente. Foram identificadas 12.175 solicitações de hemoderivados no ano de 2005. O hemocomponente mais solicitado foi o concentrado de hemácias (6.578 solicitações), seguido do concentrado de plaquetas (4.133), plasma fresco (1.296) e do crioprecipitado (168). As perdas representaram 2,96% dos eventos. As três áreas foram responsáveis por 59,77% das solicitações de hemocomponentes. A internação clínica apresentou 85,57% de solicitações em conformidade com o protocolo, seguida da CTI com 81,4% e da cirurgia com 71,42%. A maioria das solicitações de hemocomponentes esteve de acordo com o protocolo da instituição no ano de 2005. A internação clínica teve o perfil mais adequado de solicitações no ano de 2005, seguida do CTI e, por fim, da internação cirúrgica.

The use of blood-based products in a tertiary hospital is elevated so control and rational use is necessary. Many hospitals have protocols for the use of blood components. The aim of this work was to describe the epidemiological characteristics of blood-based product requests and their adjustment to the protocol of Hospital de Clínicas in Porto Alegre. Using a cross-sectional retrospective study, we analyzed the frequencies and indications of transfusion requests in 2005 in three different sectors (Clinical, Surgical and Intensive Care Unit). Each transfusion request was analyzed according to the current protocol. A total of 12,175 transfusion requests were analyzed. Requests for blood (6,578) were the commonest, followed by platelets (4,133), fresh frozen plasma (1296) and cryoprecipitate (168). Lost data represented 2.96% of the total events. The three sectors were responsible for 59.77% of blood-based product requests. The Clinical Sector requested 85.57% of its transfusions satisfactorily, followed by the ICU (81.4%) and finally the Surgical Sector (71.42%). The majority of transfusion requests in HCPA in 2005 were adequate according to the protocol. The Clinical Sector had the best request profile, followed by the ICU and the Surgical Sectort.